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Diseases

Genetic and Rare Diseases Information Center (GARD)

Pigmented villonodular synovitis


Other Names for this Disease
  • Localized pigmented villonodular synovitis
  • Diffuse pigmented villonodular synovitis
  • Tenosynovial giant cell tumors
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Your Question

I have just received a diagnosis of pigmented villonodular synovitis. Can you tell me more about this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is pigmented villonodular synovitis?

Pigmented villonodular synovitis (PVNS) is a disease in which the tissue lining the joints and tendons in the body (synovium) grows abnormally. It is characterized by a noncancerous mass or tumor. There are two types of PVNS: the local or nodular form (where the tumor involves the tendons that support the joint, or in one area of the joint) and the diffuse form (where the entire lining of the joint is involved). Symptoms might include: pain, limitation of movement, and locking of the joint. In some cases, the normal joint structure can be destroyed. The knee is most commonly affected by this condition, though it can occur in other joints such as the hip, shoulder, elbow, ankle, wrist, and rarely the jaw. The average age of diagnosis for this condition is 35 years. The cause of PVNS is grossly unknown. Treatment involves surgery to remove the tumor and damaged portions of the synovium.[1][2][3]
Last updated: 4/4/2016

What causes pigmented villonodular synovitis?

The exact cause of pigmented villonodular synovitis (PVNS) is unknown.  Some doctors believe that it is similar to arthritis, arising from swelling (inflammation) of the joint tissue. Others believe it develops like a tumor, caused by cells growing and multiplying more quickly than usual.[4] The association between a history of trauma and the development of  PVNS is unclear. One study found that 56% of individuals with PVNS had a history of previous trauma, while other studies have found a much lower incidence.[3] 

There have been studies suggesting that PVNS could be caused by specific genetic changes in the cells lining the joint. More studies are needed to research this association. [5]
Last updated: 4/5/2016

How might pigmented villonodular synovitis be treated?

Pigmented villonodular synovitis is first treated with surgery to remove as much of the abnormal tissue growth as possible.  The type of surgery depends on the location and extent of the disease within the joint.  Radiation therapy is sometimes used to treat this condition if surgery is not an option, or if the condition returns (recurs) after an initial surgery.[4][1]
Last updated: 4/5/2016

How common is pigmented villonodular synovitis?

Pigmented villonodular synovitis is rare, affecting two in a million people.[4]
Last updated: 11/28/2011

References
Other Names for this Disease
  • Localized pigmented villonodular synovitis
  • Diffuse pigmented villonodular synovitis
  • Tenosynovial giant cell tumors
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.